POS1297 PULMONARY INVOLVEMENT IN VERY EARLY SYSTEMIC SCLEROSIS (VEDOSS): REPORT FROM A SINGLE CENTER
Authors: M. Neto, A. Maduro, A. Saraiva, J. A. P. Da Silva, T. Santiago, M. J. Salvador
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Previous work has described interstitial lung disease (ILD) in very early systemic sclerosis (VEDOSS) <sup>[1]</sup>, but its progression over time is unknown. This can contribute decisively to the delineation of screening and monitoring strategies. <h3>Objectives</h3> To evaluate the prevalence of ILD and its course in a cohort of patients who persist in a VEDOSS state, followed in a tertiary systemic sclerosis unit, over 10 years. <h3>Methods</h3> Retrospective observational study of patients persistently fulfilling VEDOSS criteria <sup>[2]</sup>, with sequential measurement of diffusing lung capacity for carbon monoxide (DLCO) and forced vital capacity (FVC), at 0, 1, 5 and 10 years. Chest CT at baseline was considered, when available. A DLCO or FVC &lt;80% of predicted values was considered indicative of lung involvement <sup>[1]</sup>. A decline of FVC or DLCO of ≥5% and ≥15% in any of the time intervals was considered clinically relevant <sup>[3]</sup>. Demographic, clinical, and immunological variables at baseline were taken as potential independent variables, including age, gender, disease duration, arthritis, capillaroscopy pattern, and autoantibody profile. Pearson χ2 test, Fisher's exact test or independent sample t-test were used for independent measures as appropriate. Repeated-measures ANOVA was used to compare variables over time. <h3>Results</h3> 30 patients (86,7% women, mean baseline age 46,9±12,87 yrs) were included; median time from first symptoms to diagnosis was 4,4 (IQR 7,6) yrs. At baseline, 4 patients had mildly decreased DLCO values (60-79% of predicted), results being normal in all remaining persons (Table 1). During the first year of follow-up, a ≥5% decrease in FVC was observed in 6/30 patients (20%) and a DLCO decrease of ≥15% in 2/30 (6,7%). There was a non-significant trend for a higher probability of FVC and DLCO decrease in males vs females (M:50,0% vs F:15,3%F and M:25,0% vs F3,8%, respectively). None of the other variables was associated with FVC or DLCO decrease. Pulmonary tests were available for the 25 and 5 patients who reached 5 and 10 years of follow-up. No association was found between any of the clinical variables and significant change of FVC and DLCO at these time points. Mean values of FVC and DLCO consistently declined overtime, without reaching statistical significance (Table 1). The incidence of significant change in FVC and DLCO between contiguous evaluations is represented in Figure 1. There is a non-significant trend for a higher probability of decline as follow-up time increased. <h3>Conclusion</h3> Our study provides data on the long-term assessment of FVC and DLCO in VEDOSS patients. There was a relevant decrease in FVC during the first year in 20% of the patients, and 20% had impaired DLCO (&lt;80%) at 5 years, which suggests that a subset of patients who persist in VEDOSS state overtime still endure significant subclinical pulmonary involvement. <h3>References</h3> [1]Valentini G, Vettori S, Cuomo G, et al. Early systemic sclerosis: short-term disease evolution and factors predicting the development of new manifestations of organ involvement. Arthritis Res Ther 2012; 14: R188. 20120817. DOI: 10.1186/ar4019. [2]Avouac J, Fransen J, Walker UA, et al. Preliminary criteria for the very early diagnosis of systemic sclerosis: results of a Delphi Consensus Study from EULAR Scleroderma Trials and Research Group. Ann Rheum Dis 2011; 70: 476-481. 20101115. DOI: 10.1136/ard.2010.136929. [3]Hoffmann-Vold AM, Allanore Y, Alves M, et al. Progressive interstitial lung disease in patients with systemic sclerosis-associated interstitial lung disease in the EUSTAR database. Ann Rheum Dis 2021; 80: 219-227. 20200928. DOI: 10.1136/annrheumdis-2020-217455. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.